L5- Pulmonary Pathology III Flashcards
what are the 2 ways to classify Asthma
- based on prior sensitization to allergen: atopic, non-atopic
- based on triggers: drugs, occupational, environmental, seasonal, exercise
Atopic Asthma:
(1) mechanism
(2) triggers
(3) age of onset
(4) FHx
(5) skin tests
(6) PMHx
1- type I hypersensitivity, IgE 2- environmental: dust, pollen, dander, food 3- childhood 4- yes, FHx present 5- immeadiate wheal and flare 6- allergic rhinitis, eczema
Non-atopic Asthma:
(1) mechanism
(2) triggers
(3) age of onset
(4) FHx
(5) skin tests
(6) PMHx
1- bronchial hyper-irritability
2- viral respiratory infections, SO2, NO2, etc
3- less common, begins anytime
4- less common to have FHx
5- no skin test results
6- less common to have certain PMHx components
Atopic asthma is a (1) type of reaction or body response. The (2) will be inhaled and elicit a response from (3) cells in (4) type people. (3) will release the following, (5), in order to cause (6).
1- type I hypersensitivity (IgE mediated) 2- allergen 3- TH2 cells 4- genetically predisposed individuals 5- IL-4, IL-5, IL-13 6- IgE mediated mast cell degranulation
list the 3 most important CKs in Atopic Asthma pathogenesis, include their functions
(type I hypersensitivity reaction, IgE mediated, all released from TH2 cells)
- IL-4: stimulates IgE production
- IL-5: activates eosinophils
- IL-13: stimulates mucus production + promotes IgE production from B-cells
All are used in order to promote IgE coating of mast cells => degranulation
atopic asthma pathogenesis is split into (1) which presents at (2) time, and then (3) presenting at (4) time; this phenomenon is due to (5)
1/2- early phase, minutes
3/4- late phase, 4-24 hrs
5- two separate waves of mediators
describe the components of the early phase of Atopic Asthma pathogenesis
(1st wave of mast cell degranulation, initiates in minutes)
- bronchoconstriction (direct vagal nerve stimulation)
- inc mucus production
- vasodilation => inc permeability
- further leukocyte recruitment
describe the components of the late phase of Atopic Asthma pathogenesis
(2nd wave of mast cell degranulation, initiates 4-24 hrs later)
- activation of eosinophils, PMNs, T-cells
- epithelial activation –> TH2 cell and eosinophil recruitment
Non-atopic asthma is usually caused by (1) and or (2). (1) will induce (3) which in turn will (4) in response to (2). (5) and (6) are critical similarities to atopic asthma.
1- secondary to viral infections 2- inhaled irritants: SO2, NO2, O3 3- virus induced mucosal damage 4- lower threshold of sub epithelial vagal receptors 5- inflammatory mediators 6- Tx
(1) is the most common drug observed in drug-induced asthma. This results from (1) inhibiting (2) production and therefore increasing (3) production which are responsible for the asthma reaction, specifically (4). This will present as (5) clinically.
1- aspirin 2- TXs, PGs (dec) 3- LTs (inc) 4- LT mediated bronchoconstriction 5- recurrent rhinitis, nasal polyps, urticaria, bronchospasm
list common triggers found in Occupational Asthma
fumes, organic dusts, chemical dusts, gases: epoxy resins / plastics, wood, cotton, platinum, toluene
*note- asthma attacks follow repeated exposure
list the 2 main features of the gross appearance of asthma
- occlusion of airways by thick mucus
- lung hyperinflation (due to obstruction)
Asthma Histology:
- (1) fluid changes
- (2) epithelial changes
- (3) observed in bronchial lumen
- inc in total (4) cells
- inflammation => inc (5) cells
- (6) changes to bronchial smooth muscle cells
- (7) changes to basement membrane
1- edema
2- patchy necrosis
3- mucus plugs with Curschmann spirals, Charcot Leyden crystals, eosinophils
4- goblet cells
5- eosinophils
6- SM hypertrophy and hyperplasia
7- thickened BM // sub-basement membrane fibrosis
define:
(1) Curschmann spirals
(2) Charcot Leyden crystals
1- whorls of shed epithelium (necrotic cells)
2- collections of crystalloids formed by Protein Galactin-10 via eosinophils
Asthma is defined as (1- include Sxs + its frequency) clinically. (2) is progressively trapped in lung because of (3). Attacks are considered (4) and last for (5).
1- recurrent sudden attacks of dyspnea, chest tightness, expiratory wheezing/ronchi, cough with thick sputum
2- air –> progressive hyperinflation
3- (air trapped distal to) mucus plug in bronchi
4- episodic (nights, early mornings)
5- hrs, subsiding spontaneously or with therapy
define status asthmaticus
- severe, prolonged asthma attack
- non-responsive to therapy
- NEEDS emergency management
Physical Exam for Asthma:
- (1) RR change
- (2) on auscultation
- (3) spirometry changes
1- tachypnea
2- expiratory (mostly) wheeze –> indicates obstruction
3- FEV1 <30%, inc all lung volumes, dec FEV1:FVC
Laboratory Analysis for Asthma:
- (1) seen in serum
- (2) seen on ABG
- (3) seen in sputum
1- eosinophilia
2- hypoxia, hypercapnia, respiratory acidosis
3- Curschmann spirals, Chracot Leyden crystals, eosinophils
Eosinophilic Granuloma, aka (1), is a disease most found in (2) people. Patients usually present with (3) symptoms because of (4) changes in the lung. (5) are the ‘culprit cells’ and have (6) characteristic on histology.
1- pulmonary histiocytosis X / pulmonary Langerhan cell histiocytosis (PLCH)
2- smokers (cessation is apart of Tx)
3- dyspnea, cough
4- interstial nodular and fibrotic disease –> obstructive and restrictive changes on spirometry
5- Langerhans cells (not eosinophils)
6- Birbeck granules on EM
define the 3 components of bronchiectasis
- *permanent dilation of bronchi/bronchioles
- -> destruction of supporting tissue
- -> due to or associated with chronic necrotizing infection
what are the 3 possible etiologies/causes of bronchiectasis
- obstruction (tumor, foreign body) leading to localized bronchiectasis
- congenital / hereditary conditions (CF, immunodeficiency, Kartagener’s)
- Necrotizing/Suppurative pneumonia (virulent species: S. aureus, Klebsiella spp.)
list the few steps of Bronchiectasis Pathogenesis
1) Obstruction hampers clearance => pooling
2) secondary infection
3) damaged / weakened bronchial walls
4) bronchiectasis
describe the gross appearance of bronchiectasis
-marked dilated bronchi, filled with purulent mucus
- dilation can be followed to pleural surfaces
- dilated bronchi appear as cysts filled with mucopurulent secretions
Microscopic appearance of Bronchiectasis:
- (1) initial bronchial wall changes
- (2) later bronchial wall changes
- (3) bronchial epithelial changes
- (4) are possibly present, and (5) are usually positive in bronchiectasis
1- intense acute/chronic inflammatory exudate + necrotizing ulceration
2- fibrosis on bronchial walls –> bronchiolitis obliterans
3- squamous metaplasia
4- abscesses
5- cultures are usually positive
list the presenting symptoms of bronchiectasis
-*severe persistent productive cough with foul smelling sputum (infection is common determinant factor / trigger)
- dyspnea, hemoptysis
- clubbing, cyanosis
list the complications stemming from bronchiectasisi
- brain abscess (seeding microbe from lungs)
- cor pulmonale
- pulmonary HTN
- amyloidosis
Kartagener Syndrome is apart of the (1) group of disorders. It is inherited in (2) fashion and is a disorder in (3) which impairs (4).
1- primary ciliary dyskinesia
2- AR
3- ciliary microtubules
4- impairs mucociliary escalator (clearance of lung secretions)
Kartagener Syndrome, a (1) type disorder, results in persistent (2) leading to (3). There is also decreased (4) in males leading to (5).
1- primary ciliary dyskinesia
2/3- lung infections –> bronchiectasis
4/5- dec spermatazoa motility –> sterility
list the main symptoms of components of Kartagener Syndrome
- bronchiectasis
- chronic sinusitis
- (1/3 of people) situs inversus
Restrictive Lung Disease involves the (1) of CT and the hallmark feature is (2).
1- inflammation and fibrosis of pulmonary CT
2- involvement of interstitium
define the 3 synonyms for interstitial lung disease
Infiltrative: cellular and non-cellular infiltrates w/in alveolar septa/alveoli
Restrictive: reduced TLC + normal/reduced expiratory flow rate
DPLD (diffuse parenchymal): involves intra-alveolar space
list the components of lung interstitium
-basement membrane of endothelium and epithelium
- collagen, elastin
- fibroblasts, scattered mononuclear inflammatory cells (alveolar macrophages)
the etiopathogenesis for restrictive lung diseases is….
largely unknown
define alveolitis
damage to pneumocytes and endothelial cells (via inflammation)
Most restrictive lung disease involves (1) infiltration in the (2) part of the lung where (3) mediate and stimulate (4). (4) will lead to decreased (5) and increase (6) of the lung.
1- leukocyte 2- interstitium 3- CKs 4- interstitial fibrosis 5- dec lung compliance (expansion) 6- inc elasticity (recoil)
5/6 => dec lung expansion upon inspiration
The main feature of restrictive lung disease is (1) which leads to decreased (2) of the lung which presents as (3) symptom. (1) and damage to interstitium leads to (4) and presents as (5) symptoms.
1- fibrosis 2- lung compliance / stiff lung (--> inc work to breath) 3- dyspnea 4- VQ mismatch --> hypoxia 5- tachypnea, cyanosis
describe spirometry results of Restrictive Lung Disease
- dec in all lung volumes
- dec in flow rates
- normal to inc FEV1:FVC
- dec DLCO (diffusing capacities)
describe CXR results in Restrictive Lung Disease
bilateral infiltration:
- nodular/reticular
- ground-glass shadows / infiltrative
The main complications of restrictive lung disease is progression to….
- cor pulmonale
- pulmonary HTN
IPF = (1) / (2):
- (3) etiology
- males (=/>) females
- (5) age group
- (6) prognosis
1- idiopathic pulmonary fibrosis 2- UIP, usual interstitial pneumonia 3- unknown 4- M > F 5- 2/3 > 60 y/o (mean is 40-70 yrs) 6- poor (5 yr survival is 20%, mean survival is <3 yrs)
IPF/UIP has progresses to (1) leading to (2). Its progression is described as (3). (4) is the main treatment protocol.
(idiopathic pulmonary fibrosis, usual interstitial pneumonia) 1- bilateral fibrosis 2- severe hypoxia, cyanosis 3- relentless (=> poor prognosis) 4- lung transplantation
IPF and UIP will have insidious onset of (1) and (2). (3) will be present on auscultation. In the late stages, the following will become more apparent: (4).
(idiopathic pulmonary fibrosis, usual interstitial pneumonia) 1- dry cough 2- exertional dyspnea 3- crackles on inspiration 4- cyanosis, cor pulmonale, clubbing
describe the radiographic appearance of IPF/UIP (indicate which processes / diseases must be ruled out)
(idiopathic pulmonary fibrosis, usual interstitial pneumonia)
- restrictive pattern: inc interstitial markings (fibrosis) + Honey Comb appearance
- fibrosis is mainly in lower lobes bilaterally
describe the gross appearance of the lung in IPF/UIP
(idiopathic pulmonary fibrosis, usual interstitial pneumonia)
- cobble-stoned pleural surface
- honeycomb cysts
-firm, fibrotic parenchyma with lower lobe and subpleural accentuation
IPF/UIP Histology:
- (1) hallmark feature
- (2) is the difference of (1) in different places, therefore (3) and (4) by co-exist
- worse in (5) areas
(idiopathic pulmonary fibrosis, usual interstitial pneumonia)
1- patchy interstitial fibrosis
2- temporal (time) and geographic (spatial) heterogeneity
3- mature fibrosis
4- young fibroblast foci
5- accentuation beneath pleura and along interlobular septa
Note- some areas are spared, normal lung
describe the honeycomb changes in IPF/UIP
(idiopathic pulmonary fibrosis, usual interstitial pneumonia)
- cystic spaces lined by type II pneumocytes / respiratory epithelium
- dense fibrosis destroys architecture of parenchyma –> cysts
